1/104. Iridocorneal melanoma associated with type 1 neurofibromatosis: a clinicopathologic study. OBJECTIVE: A clinicopathologic study of an iridocorneal melanoma associated with type 1 (peripheral) neurofibromatosis is presented. DESIGN: Case report with clinicopathologic correlation. PARTICIPANT: A 32-year-old white woman with type 1 neurofibromatosis presented with long-standing blindness of her right eye due to diffuse intrastromal brown corneal discoloration. INTERVENTION: The patient underwent penetrating keratoplasty and the corneal button was inspected. RESULTS: Histopathologic evaluation of the corneal button after penetrating keratoplasty revealed an intrastromal mixed-type malignant melanoma, which stained positively with HMB-45 and S-100 protein and spared the corneal epithelium and limbus. The corneal graft remained transparent, with best-corrected visual acuity of 20/30. Twenty-two months after surgery, the tumor involved the anterior chamber angle and the iris. Three years later, it caused refractory glaucoma necessitating enucleation. The iris tumor did not extend beyond the iris-lens diaphragm and showed the same cytologic features as the corneal stromal tumor. CONCLUSION: To our best knowledge, this is the first report of iridocorneal melanoma associated with peripheral neurofibromatosis. The location of the tumor in the deep corneal stroma, without initial macroscopic involvement of the angle or iris, may suggest that the corneal portion of the tumor may have developed "in situ" rather than as an extension of iris melanoma. The common origin of melanoma cells and schwann cells from the neural crest and the proliferation of the schwann cells in neurofibromatosis provides additional support for this hypothesis. ( info) |
PURPOSE: To report a case of metastatic renal cell carcinoma with involvement of the iris and bulbar conjunctiva. methods: Case report. A 70-year-old man without known systemic disease developed an iris mass in his left eye. Iridocyclectomy was performed to remove the iris mass. RESULTS: Histopathologically, the iris mass was consistent with metastatic renal cell carcinoma. Further evaluation disclosed a mass of the left kidney. The patient underwent a left nephrectomy and was found to have renal cell carcinoma, with focal penetration into the renal capsule. One month after the nephrectomy, he developed a highly vascular nodule of the left bulbar conjunctiva. An excisional biopsy was performed, and histopathology disclosed an additional focus of renal cell carcinoma. CONCLUSIONS: iris and conjunctival involvement may be a clinical manifestation of renal cell carcinoma. Renal cell carcinoma should be considered in the differential diagnosis of a fleshy, vascular iris and a conjunctival nodule. ( info) |
3/104. Clinical and histopathologic findings of iris nevus (Cogan-Reese) syndrome. PURPOSE: To report a case of Cogan-Reese syndrome. METHOD: Case report. A 37-year-old man presented with Cogan-Reese syndrome. RESULTS: visual acuity was 0.5 in the right eye and 1.0 in the left eye. There were corneal edema and pigmented nodules on the anterior surface of the iris, iris atrophy and ectropion uvea in the right eye. The intraocular pressure was 42 mmHg in the right eye and there was glaucomatous optic atrophy of the optic disk. trabeculectomy with mitomycin C has been performed as the intraocular pressure did not decrease with the maximum medical treatment. Electron microscopic examination of the trabeculum and the iris tissue revealed a lot of melanocytic cells in the stroma. CONCLUSION: trabeculectomy with mitomycin C might be effective in Cogan-Reese cases with glaucoma resistant to medical treatment. ( info) |
4/104. Tapioca melanomas of the iris: immunohistology and report on two cases. BACKGROUND: Tapioca-like tumors are rare, and their benign or malignant nature is obscure without histological work-up. We report on the clinical and histological features of different types of tumors in two patients. CASE REPORT: Two patients aged 17 and 45 years presented with brownish iris masses increasing in size. Full-thickness en bloc excision of melanocytic tumors (5.5 mm and 7.0 mm in diameter) was carried out. Histological work-up revealed a nevus cell nevus in the young patient and an epithelioid malignant melanoma in the middle-aged patient evolving from the ciliary body. Staining for HMB-45 was marked in both tumors, for S-100 low in the nevus and marked in the melanoma, and for p53 negative in the nevus and positive in the melanoma. Ki67 stains were negative. CONCLUSION: We propose that excision of anteriorly located pigmented tumors that increase in size is indicated in order to determine whether they are benign or malignant. In these case reports, tapioca-like tumors include a benign and a malignant entity. To our knowledge, a nevus cell nevus presenting as a tapioca-like tumor has not previously been described. Curative surgery and histological and immunohistological evaluation are required to characterize the malignant potential of these tumors and the prognosis. ( info) |
5/104. adenoma of the iris pigment epithelium: a report of 20 cases: the 1998 Pan-American Lecture. BACKGROUND: adenoma of the iris pigment epithelium (IPE) is an uncommon lesion that can simulate iris or ciliary body melanoma, melanocytoma, and pigment epithelial cyst. OBJECTIVES: To evaluate the clinical and pathological features and prognosis of adenoma of the IPE in patients managed by us and to elucidate the features that help to differentiate this tumor from iris melanoma and other similar conditions. patients AND methods: The medical records of 20 patients with adenoma of the IPE were reviewed, and the clinical and histopathologic features were tabulated. RESULTS: Ten patients were male and 10 were female, with a mean age of 60.0 years (range, 11-85 years). All patients were referred because of suspected iris or ciliary body melanoma. All lesions were solitary and unilateral. Sixteen were located in the peripheral iris; 2, in the midzone; and 2, near the pupillary margin. Clinically, all tumors were abruptly elevated, all but 1 were dark gray to black, and all had a smooth, but sometimes multinodular, surface. The tumors caused thinning or complete effacement of the overlying iris stroma, but they did not directly involve the stroma. They typically blocked light with transillumination. On ultrasound biomicroscopy findings, adenoma of the IPE shows a solid tumor pattern, sometimes with small cystoid spaces. The tumor was managed by local resection in 2 patients and observation in 18, all of whom have been stable, with follow-up ranging from 6 months to 9 years. Histopathologic examination revealed a tumor originating in the IPE consisting of cords of pigment epithelial cells separated by septae of connective tissue. CONCLUSIONS: adenoma of the IPE usually has characteristic features that should differentiate it from iris melanoma, ciliary body melanoma, iris melanocytoma, and iris cyst. adenoma of the IPE is a benign tumor that may remain relatively stable for years. ( info) |
6/104. Multifocal intraocular malignant melanoma: report of two cases and review of the literature. PURPOSE: To describe two eyes from two patients with multifocal primary intraocular melanoma. DESIGN: Two case reports. methods: The history and histologic findings in the enucleated eyes of two patients with multifocal intraocular melanoma are described in comparison to previously reported cases. MAIN OUTCOME MEASURES: Pathologic examination of enucleated eyes. RESULTS: One of the two eyes contained mixed cell type melanomas, and one eye contained spindle cell type melanomas. Examination of serial sections showed no continuity between the intraocular melanomas. There were no associated ocular or systemic conditions with the multifocal intraocular melanomas. CONCLUSIONS: Multifocal primary intraocular melanoma is rare. There is no known predisposing factor to this condition. ( info) |
7/104. Photocoagulation of iris nevus to control recurrent hyphema. PURPOSE: To report a case of recurrent hyphema caused by an iris nevus that was successfully treated with photocoagulation. METHOD: Case report. In a 30-year-old woman with recurrent hyphema secondary to an iris nevus, photocoagulation was applied to the iris nevus. RESULTS: Before photocoagulation of the iris nevus, the patient had increasing frequency of hyphema episodes. After treatment, no hyphema episodes occurred during 13 months of observation. CONCLUSION: Photocoagulation of the surface of an iris nevus may prevent recurrent hyphema. ( info) |
8/104. A case for electronic manipulation of medical images? Two ophthalmic case histories requiring differing techniques to document the long-term outcomes of ocular tumours are presented. In the first case, after documenting a treated choroidal melanoma it was necessary to intervene with digital manipulation to enhance the images for clinical interpretation. Conversely, after documenting an iris tumour over an eight-year period, it was considered that the original images were suitable for interpretation without intervention. This raises the issue of images presented as legal evidence when digital technology is used to manipulate and enhance the images. Are these images admissible as evidence in court, and should the original data also be archived to prevent conflicting interpretations by the legal system? In light of The House of Lords recommendations on digital images as evidence, it is suggested that both the manipulated and original images are archived to avoid debate on the extent to which an image has been altered. ( info) |
9/104. Malignant melanoma of the conjunctiva with intraocular extension. Intraocular extension of a malignant melanoma of the conjunctiva is a rare entity. A 75-year-old woman underwent repeated surgery after receiving the diagnosis of a multilocular recurrent malignant melanoma arising from a primary acquired melanosis. Treatment included 2 lamellar sclerokeratectomies and percutaneous radiotherapy. Five years after initial surgery, intraocular extension of the melanoma was observed, and enucleation was performed. Findings from histopathological examination revealed a malignant melanoma occupying part of the ciliary body, the trabecular meshwork, and the iris. Eyes with recurrent malignant melanoma of the conjunctiva should be carefully monitored for intraocular extension. Deep excision of conjunctival melanoma, including lamellar sclerokeratectomy, may abolish the natural barrier against intraocular extension of malignant melanomas of the conjunctiva. ( info) |
iris varix is rare and little is known about its clinical characteristics. We treated a thrombosed iris varix that simulated an iris melanoma. A 53-year-old man developed a dark brown iris mass and hyphema in his left eye. Ultrasound biomicroscopy revealed a circumscribed mass of the iris stroma. Because of suspicion for melanoma, it was removed by sector iridectomy. Histopathologic examination disclosed an extensive focus of stromal hemorrhage, partially surrounded by endothelial cells that showed immunoreactivity to vascular markers. The histopathologic diagnosis was thrombosed iris varix. iris varix is a rare condition that should be included in the differential diagnosis of iris melanoma. ( info) |